OBJECTIVE

To present a long-term follow-up report of a 10-year-old male with optic nerve glioma who underwent surgical removal and postoperative chemotherapy.

METHODS

Case report.

RESULTS

A 10-year-old Filipino boy was referred to a tertiary institution for a five-year history of progressive right eye proptosis with vision loss. Pertinent findings included right eye proptosis, lagophthalmos, and limited elevation and adduction. He also had several hyperpigmented lesions on the abdomen and upper torso. Vision on the right was no light perception, with a relative afferent pupillary defect, exposure keratopathy, and optic nerve pallor. Vision on the left eye was 20/20 with a temporal visual field defect. Cranial and orbital computed tomography (CT) scan showed a circumscribed enhancing mass within the right intraconal space with widened right optic nerve canal. Additional magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass diffusely involving the intraorbital and intracanalicular segments of the right optic nerve suspicious for optic nerve glioma. He underwent excision of the orbital portion of the mass via lateral orbitotomy. Histopathology showed pilocytic astrocytoma. Eight cycles of chemotherapy with carboplatin and vincristine was completed. Significant improvement of globe position and resolution of ocular exposure was achieved postoperatively with residual right ptosis. These findings remained stable at six years after treatment.

CONCLUSION

Optic nerve gliomas with intracanalicular and chiasmal extension can be managed with surgical removal of the orbital component and postoperative chemotherapy. This can result in improvement of proptosis and long-term remission.

Human ; Male ; Child: 6-12 Yrs Old ; Optic Nerve Glioma ; Neurofibromatosis 1 ; Pilocytic Astrocytoma ; Astrocytoma ; Chemotherapy ; Drug Therapy

Objective: To describe a case of adult-onset foveomacular vitelliform dystrophy (AOFVD). Method: This is a case report. Results: A 22-year-old female presented with painless blurring of vision and metamorphopsia 3 days prior to consultation. There were 2 similar episodes in the past that spontaneously resolved after 2 to 4 weeks. Visual acuity (VA) was 20/50 in the right eye (OD) and 20/40 in the left (OS), both best corrected to 20/25. Dilated-fundus examination revealed a discrete area of mixed hypoand hyperpigmentation 1 disc diameter over the fovea in OD and a solitary round hypopigmented lesion with a hyperpigmented border 3 to 4 disc diameters on the fovea in OS. Fluorescein angiography (FA) revealed an area of hyperfluorescence surrounded by a rim of hypoflourescence in OD and an area of blocked fluorescence with subtle hyperfluorescence superior to the lesion in OS, both of which did not increase in size and intensity toward the late phases. Optical coherence tomography (OCT) revealed neurosensory detachment in both eyes. Electrooculogram (EOG) was normal with Arden ratio of 0.91. VA returned to 20/25 in both eyes, and repeat fundus photography showed no change in the characteristics of the lesions. Conclusion Differential diagnosis of a hypopigmented macular lesion in the young with self-limited blurring of vision should include AOFVD. FA, OCT, and EOG can help distinguish AOFVD from Best’s disease or other similar macular conditions.
Vitelliform Macular Dystrophy ; Bestrophins ; Peripherins